Disease Outcomes
Patients were followed with an average duration of 30.5 ± 44.5 months (median, 12 months; range, 9 days to 167.5 months). Thirteen patients (38%) died by the end of follow-up. Systemic fungal infection, sepsis, and intracranial complications of AIFR were the causes of death in 9 patients (26%); other complications of the underlying disease, including non-remission and secondary infections, accounted for the remaining four deaths (12%).
The mean survival time of patients who died of AIFR was 98 ± 144 days (median, 36 days; range, 14-462 days). In a univariate analysis, diagnosis using panfungal PCR (p=.04 ) and treatment with novel antifungal agents (p=.017 ) were associated with increased rates of AIFR-specific survival. Remission status was associated with overall survival (p=.008 ). However, a regression analysis using the Cox-proportional hazard model to control for confounding effects did not identify any significant variable associated with survival (Table 2 ).
The overall and disease-specific survival rates increased throughout the study period. The last patient to die of AIFR was diagnosed in February 2012. Since then, no other patient has died of the disease. Moreover, since April 2013 only one patient has died of his underlying malignant disease (Figure 1 ). Increased survival was evident specifically among patients with mucormycosis. Seven of these patients were diagnosed before 2012 and the remaining five between 2013-2020. Four patients (33%) died from complications related to mucormycosis, all diagnosed before 2012. Of note are two patients with mucormycosis who survived despite an aggressive disease involving, in one patient, the orbit, skull base and dura, and in the second the hard palate and skull base. Both patients received rapid empirical treatment with ambisome as soon as AIFR was suspected, and underwent multiple aggressive surgical procedures, including resection of the skull base, the hard palate and orbit.